Risk factors associated with invasive orthopaedic interventions in males with haemophilia enrolled in the Universal Data Collection program from 2000 to 2010.

BACKGROUND: Invasive orthopaedic interventions (IOI) are often used to control recurrent haemarthrosis, pain and loss of joint function, in males with haemophilia (Factor VIII and Factor IX deficiency). AIM: Identify risk factors associated with IOIs in males with haemophilia enrolled in the Universal Data Collection (UDC) surveillance program from 2000 until 2010. METHODS: Data were collected on IOIs performed on patients receiving care in 130 haemophilia treatment centers in the United States annually by health care providers using standardized forms. IOIs included in this study are as follows: 1) synovectomy and 2) arthrodesis or arthroplasty (A/A). Information about potential risk factors was obtained from the preceding UDC visit if available, or from the same visit if not. Patients with no reported IOI at any of their UDC visits were the reference group for the analysis. Multivariate analyses were conducted to identify independent risk factors for synovectomies and arthrodesis/arthroplasty. RESULTS: Risk factors significantly associated with the two IOI categories were age, student status, haemophilia severity, number of joint bleeds within the last 6 months, HIV or hepatitis C (HCV) status. Multivariate analyses showed patients on continuous prophylaxis were 50% less likely to have had a synovectomy and were 40% less likely to have an A/A. CONCLUSIONS: This study shows modifiable risk factors, including management of bleeding episodes with a continuous prophylactic treatment schedule are associated with a decreased likelihood of IOIs in males with haemophilia.

Meta-analysis: outcomes of total knee arthroplasty in the haemophilia population.

INTRODUCTION: People with haemophilia (PWH) experience end stage joint disease as a result of repeated hemarthrosis, commonly leading to total knee arthroplasty (TKA). AIM: The goal of this meta-analysis is to calculate expected outcomes for range of motion (ROM), functional mobility, and complication rates in PWH following TKA. METHODS: Studies published between 1980 and 2015 were identified. INCLUSION CRITERIA: PWH having TKA, reporting Hospital for Special Surgery Knee Score or Knee Society Score, knee ROM, and incidence of complications for more than 5 TKAs. Inhibitor status, haemophilia severity and HIV status were not criteria for inclusion or exclusion. Meta-analysis was performed using mean, standard deviation, or P-value data to create effect sizes (ES) and 95% confidence intervals for each variable. RESULTS: Twenty studies met inclusion criteria; ten had sufficient data for meta-analyses. A total of 336 TKAs in 254 PWH were analysed with mean follow-up of 6.3 years. Statistically significant ROM improvements were found with 9.72° improvement of flexion contracture (-0.73 effect size (ES) (-0.91 to -0.56)), and 15.69°increase into flexion (0.63 ES (0.34-0.91)). Knee scores showed statistically significant improvements: clinically, 37.9 point increase (3.21 ES [1.79-4.63]) and functionally, 13.50 point increase (1.50 ES [0.80-2.21]). A 31.5% complication rate was calculated with 106 reported in 336 TKAs. CONCLUSIONS: TKA is an effective procedure for improving ROM and decreasing functional deficits resulting from haemophilic arthropathy. Knee score data shows TKA improves overall function. This study guides clinicians regarding outcome expectations post-TKA in PWH.

Declining trends in invasive orthopedic interventions for people with hemophilia enrolled in the Universal Data Collection program (2000-2010).

INTRODUCTION: Recurrent joint hemarthroses due to hemophilia (Factor VIII and Factor IX deficiency) often lead to invasive orthopedic interventions to decrease frequency of bleeding and/or to alleviate pain associated with end-stage hemophilic arthropathy. AIM: Identify trends in invasive orthopedic interventions among people with hemophilia who were enrolled in the Universal Data Collection (UDC) program during the period 2000-2010. METHODS: Data were collected from 130 hemophilia treatment centers in the United States annually during the period 2000-2010, in collaboration with the Centers for Disease Control and Prevention (CDC). The number of visits in which an invasive orthopedic intervention was reported was expressed as a proportion of the total visits in each year of the program. Invasive orthopedic interventions consisted of arthroplasty, arthrodesis, and synovectomy. Joints included in this study were the shoulder, elbow, hip, knee, and ankle. RESULTS: A 5.6% decrease in all invasive orthopedic interventions in all joints of people with hemophilia enrolled in the UDC program over the 11-year study period was observed. CONCLUSIONS: These data reflect a declining trend in invasive orthopedic interventions in people with hemophilia. Further research is needed to understand the characteristics that may influence invasive orthopedic interventions.

Functional outcomes following ankle arthrodesis in males with haemophilia: analyses using the CDC's Universal Data Collection surveillance project.

In persons with haemophilia (PWH), repeated ankle haemarthroses lead to pain, loss of joint range of motion (ROM), and limitations in activity and participation in society. PWH are offered ankle arthrodesis (AA) to eliminate pain. In our experience, PWH are hesitant to proceed to AA due to concerns regarding gait anomalies, functional decline and complete loss of ROM. The aim of this study was to report outcomes in ROM, assistive device (AD)/wheelchair use, activity scale and work/school absenteeism for participants in the CDC's Universal Data Collection surveillance project (UDC) pre- and post- AA. Males with haemophilia enrolled in the UDC with first report of AA (1998-2010) were selected. Descriptive statistics were calculated using data from the annual study visit pre-AA and the follow-up visit (~12-24 months) post-AA. The 68 subjects who fulfilled the criteria were: mean age 36.9 years (SD = 12.9); 85.3% white; 85.3% haemophilia A; 72% severe, 20.6% moderate; and 10.3% with inhibitor once during the study period. Mean loss in total arc of ankle motion was 17.02° (SD = 21.8, P ≤ 0.01) pre- compared to post-AA. For 61.8%, there was no change in use of AD for ambulation/mobility. For 85.3%, there was no change in use of a wheelchair. On a self-reported activity scale, 11.8% improved, 8.8% worsened and 79.4% did not change. Work/school absenteeism averaged 2.7 (SD = 6.4) pre- and 1.5 (SD = 6.4, P = 0.26) days per year post-AA. While ankle ROM was significantly reduced post-AA, for most subjects, there was no change in use of AD/wheelchair for ambulation/mobility. Physical activity was maintained and work/school absenteeism remained stable.

Outcomes in total elbow arthroplasty in patients with haemophilia at the University of California, San Francisco: a retrospective review.

Severe haemophilic arthropathy of the elbow is a significant cause of morbidity among adults with haemophilia. However, previous reports of total elbow arthroplasty (TEA) in the haemophilic population have been based on small numbers of patients with relatively short-term follow-up. The records of seven total elbow arthroplasties in six adult men with haemophilia at the University of California, San Francisco who underwent TEA over a period of 25 years were retrospectively reviewed. Type of haemophilia, age at time of TEA, HIV infection status, pre- and postoperative range-of-motion (ROM) scores, complications (including infections), need for subsequent surgical revision and functional outcomes were recorded. Four patients had severe factor VIII deficiency and two patients had severe factor IX deficiency. None of the patients had an inhibitor. The mean age at the time of surgery was 34 years (range, 22-46 years) and the mean follow-up period was 118 months (range, 37-176 months). One of the six patients had TEA in both elbows. Five of the six patients were infected with HIV. There were no immediate perioperative complications. At a mean of 19.2 months postoperatively, ROM had improved in five of seven TEAs: mean flexion had increased from 110.7° (SD = 15.0) to 120.1° (SD = 14.5), whereas mean preoperative extension increased from -44.3° (SD = 21.5) to -36.9° (SD = 27.0). One patient required a revision at 30 months because of ulnar component loosening. This same patient sustained a staph epidermidis infection and ultimate removal of the prosthesis 15 years postoperatively. At a mean of 118 months postoperatively, five of six patients continued to report reduced pain and preserved functionality, with ability to perform normal daily activities. TEA resulted in favourable results in six of seven procedures. Our findings support the viability of TEA for individuals with severe haemophilic arthropathy of the elbow, especially to reduce pain and preserve or restore functionality.